5.3.1 Flashcards
What are the three important pathways of iron once it is utilise by the mithochondria?
-Heme Synthesis
-Fe-S clusters Biogenesis
-Mithochondrial Iron Storage (ferritin)
What is the tetramer of four globin chains with heme attach to each globin chain?
-Hemoglobin
These are bonds that are located between the diamers.
-a1B2 and a2B1 bonds
What is the hormone secreted by the Hepatocytes?
-Hepcidin
What will happen if the Fe level is low?
-hepcidin will increase, recycling and absorption will also be activated. (if increase then the hepcidin will increase also but will inactivate the absorption of erythrocytes and the recycling of the macrophages and hepatocytes)
What is the most common form of anemia?
-IDA (iron dificiency anemia)
What is the good indicator of iron storage status and the first laboratory test that will result in abnormal IDA?
-Serum Ferritin
The sample of this test must be drawn early in the morning and required 12 hour fasting?
-Serum Iron
This can be measured by a hematoflourometer or by an extraction of flourescence method?
-FEP (free erythrocyte porphyrin)
Measures the maximum amount of iron that can bind to serum transferrin and an indirect measure of the protein transferrin?
-TIBC (total iron binding capacity)
How many grams of iron that the human body contain?
-4g
If the 60% of the iron stored in blood, then where’s the remaining of it stored?
-ferritin or hemosiderin
Disease that occurs when the iron stores in the body have been depleted?
-IDA
What stage of IDA that the functional iron depletes and the blood becomes microcytic and hypochromic?
-Stage 3 (Stage 1 - storage iron & Stage 2 - transport iron)
What are the clinical symptoms of IDA?
-fatigue, weakness, diziness, pica, stomatitis, glossitis, koilonychia
Clinical symptoms of IDA that is when a person is craving or eating unusual food such as starch?
-Pica
Sore tongue?
-Glossitis
Spooning of the nails?
-Koilonychia
What will show under the microscope if the blood smear we used is from a patient that has IDA?
-Ovalocytes or pencil forms of cells
This is the 2nd most common form of anemia and most common in hospitalized patients?
-ACD (Anemia of Chronic Disease)
Erythocytes during ACD cannot aquire iron salvage due to the lack of lactoferritin receptors. What is this substance?
-Lactoferritin
What influences the intestinal iron absorption and the release of iron in the macrophages?
-Hepcidin
Substance secreted bby macrophages that inhibits erythropoiesis and stimulates neutrophils to lactoferrin?
- IL 1
What substance that increases when iron storage in the body becomes low and decreases when the storage becomes high?
-Transferrin
Hepatocyte production of transferrin is regulated by?
-intracellular iron levels
What is the hallmark of sideroblasmic anemia?
-Ringed sideroblast
It is characterized by iron loading and the accumulation of ferric iron in the mithochondria due to a defect in the heme synthesis?
-Sideroblastic anemia
Cells that are iron-containing normoblast in the Bone marrow.
-sideroblast
Iron deposits in the sideroblast can be identified using what kind of stain?
-prussian blue stain
What type of sideroblast where ringed sideroblast are visible?
-Type 3
What are the cause of aquired secondary sideroblastic anemia?
-drugs and bone marrow toxins
Most common cause of sideroblastic anemia.
-alcoholism
Type of sideroblastic anemia that is associated with decrease ALA-synthase and BM erythroid hyperplasia?
-Idiopathic sideroblastic anemia
Type of sideroblastic anemia that follows afterr exposure to certain drugs and toxins?
-Secondary sideroblasstic anemia
Certain drugs that trigger secondary sideroblastic anemia.
-isoniazid
-cycloserine
-pyranizamide
What is the coenzyme that inhibits the reactions of the drugs?
-pyridoxal-5’-phosphate
What is the other name for Lead poisoning?
-Plumbism
This disease seen mostly to children due to lead-based paint?
-Lead poisoning
What is the acceptable levels of lead in children?
-15-40 ug/dl of WB
Lead Inhibits:
-Ala dehydratase or PBG synthase (most sensitive)
-Heme synthesis
-Coproporphyrinogen oxide
What is an enzyme that involves in the breakdown of RNA in reticulocytes that causes ribosomes to aggregates?
-Pyrimidine 56-nucleotidase
It is the formation of blue or black deposits of lead sulphate near the teeth.
-gum lead line
This is a inherited disorder cause by specific enzye defects necessary for the synthesis or protoporphyin resulting to decreased hemoglobin.
-Prophyrias
What is the precursor of abnormal protoporphyin?
-Prophyrins
Clinical Features of Major Porphyrias
-AIP
-CEP
-HCP
-VP
-PCT
What is the screening test of urine, red blood cells and feces for porphyrins?
-Watson-schwartz test
This is called to patient’s have bronze colored skin tht later develop to type 1 DM?
-Bronze diabetes
This result from mutations that impair the Hepcidin-ferroportin iron regulatorry system.
-Heriditary hemochromatosis
This also results from iron accumulation that causes little injury.
-Hemosiderosis
These two forms during the body first reaction to store excess iron resulting tissue damage.
-ferritin and hemosiderin
It is the iron aquisitions that exceeds the rate of loss.
-Iron Overload
What are called the cracks in the corner of the mouth?
Stomatitis
